Résumé
Myofibroma is a rare benign mesenchymal tumor of uncertain histogenesis. A six-year-old boy presented with a unilateral lower eyelid mass of six weeks' duration. MRI revealed a circumscribed mass in the inferolateral orbit with bony erosion. A systemic examination was unremarkable. Excision with histopathology revealed a partially infiltrative spindle cell tumor with bland nuclear morphology expressing smooth muscle actin and muscle-specific actin, compatible with myofibroma. Solitary myofibroma is a rare childhood orbital tumor and may clinico-radiologically closely mimic a malignancy. Histopathology and immunohistochemistry can help reach a definitive diagnosis. Systemic evaluation and close follow up are crucial in such cases.
Résumé
Abstract: This study describes a case of a 19-year-old patient with seven asymptomatic lesions on the chest, measuring between 0.5 to 1cm in diameter, with no history of trauma in the region. The immunohistochemical evaluation was positive for vimentin and smooth muscle actin, determining Dermatomyofibroma as definitive diagnosis. Dermatomyofibroma is a benign skin tumor, with a myofibroblastic origin, prevalent in young women. It usually presents as a single lesion, with very few reports of multiple lesions.
Sujets)
Humains , Femelle , Jeune adulte , Tumeurs cutanées/anatomopathologie , Myofibrome/anatomopathologie , Biopsie , Immunohistochimie , Cicatrice hypertrophique/anatomopathologie , Fibroblastes/anatomopathologieRésumé
Objective@#To investigate the clinical and histological features, diagnosis and differential diagnosis of myofibroma/myofibromatosis.@*Methods@#The clinical data and pathology features of nine cases of myofibroma/myofibromatosis were collected from August 2011 to November 2016 in Affiliated Drum Tower Hospital, Nanjing University Medical School and Children′s Hospital of Nanjing Medical University. Immunohistochemistry(IHC), PDGFRB molecular analysis and ETV6-NTRK3 gene fusion were performed and relevant literature reviewed.@*Results@#There were 7 males and 2 females, with age ranging from 3 days to 18 years (mean 5 years). The tumors were located in head and neck (eight cases) and trunk (one case). Clinically, the tumors presented as freely movable nodules. Microscopically, they appeared biphasic with alternating light- and dark-staining areas. The light-staining area consisted mainly of plump myoid spindle cells with eosinophilic cytoplasm arranged in nodules, short fascicles, or whorls.The dark-staining area was composed of round or polygonal cells with slightly hyperchromatic nuclei or small spindle cells arranged around a distinct hemangiopericytoma-like vascular pattern. IHC showed the tumor cells in the light-staining area were strongly positive for vimentin and SMA, while cells in dark-staining area were strongly positive for vimentin, and weakly for SMA. Tumor cells were negative for desmin, S-100 protein, h-Caldesmon, CD34 and STAT6. Analysis of PDGFRB mutations was performed in seven cases. Two cases showed 12 exon point mutation c. 1681 c>T(p.R561C), one case showed 14 exon point mutation c. 1998C>G (p.N666K). ETV6-NTRK3 gene fusion was not detected by fluorescence in situ hybridization in four patients under three years old. All cases were followed for 6 to 68 months, with two recurrences.@*Conclusions@#Myofibroma/myofibromatosis is an uncommon benign myofibroblastic tumor of infancy and childhood. The tumor can appear biphasic, and may show PDGFRB point mutation which is of potential diagnostic value.
Résumé
Myofibroblasts have been implicated in most fibro-contractive diseases and even in developing and specialized normal body tissues. Myofibroma denotes benign neoplasms composed of contractile myoid cells arranged around thin-walled blood vessels affecting the soft tissue, bone, or internal organs at all ages with the intraosseous variant being an uncommon entity. Histologically, these tumors are characterized by being circumscribed masses of spindle cells, which may lead to confusion in diagnosis with more aggressive soft tissue spindle cell tumors. A case is reported of a 7-year-old female patient who presented with a solitary intraosseous myofibroma in the inferolateral aspect of the left orbit. This article describes the clinical, radiographic, histopathologic, and immunohistochemical features of an intraosseous myofibroma in a female child.
Résumé
Lipoleiomyoma is an uncommon neoplasm of the uterus, composed of smooth muscles intermixed with mature adipocytes. These tumors are considered a benign variant of uterine leiomyomas. Herein, we report six cases of lipoleiomyoma experienced in our institution from January 2005 to March 2015. The patients ranged in age from 45 to 70 years; the etiology may be related to estrogen deficiency occurring after menopausal transition. Except for one lipoleiomyoma in the broad ligament, all others were found in the uterine corpus. The presenting symptoms were nonspecific, and most cases were incidentally diagnosed during surgery for other reasons. We performed preoperative imaging studies, including abdominal and pelvic computed tomography and magnetic resonance imaging. Preoperatively, four patients were diagnosed as having a pelvic mass and one patient was diagnosed as having a right ovarian mature teratoma. In one case, we found a gynecologic malignancy (cervical cancer 1A1). Histologically, there was no gross or microscopic contiguity between the lipoleiomyoma and the malignancy. Lipoleiomyomas seem to have a benign clinical course. In our study, there were no recurrences of or deaths attributed to the lipoleiomyomas during a mean follow-up period of 16.17 +/- 23.80 months.
Sujets)
Femelle , Humains , Adipocytes , Ligament large de l'utérus , Oestrogènes , Études de suivi , Léiomyome , Imagerie par résonance magnétique , Muscles lisses , Myofibrome , Périménopause , Post-ménopause , Récidive , Tératome , UtérusRésumé
The myofibroma is defined as a benign neoplasm formed by myoid contractile cells localized around the wall of thin blood vessels, it is a tumor that presents itself mostly in infancy although it may occur at any age and it is most common in head and neck; it is uncommon to be localized in the oral cavity and even less common if it is intraosseous. It may also be associated to miofibromatosis or present itself as a solitary lesion. The differential diagnosis depends on the localization and the radiographic characteristics; it would be very difficult to include, at first hand, myofibroma as an intraosseous lesion. Histopathologically, a neoplasm with a biphasic pattern formed by spindle cells in fascicles and bundles, spindle nucleus with eosinophilic cytoplasm inside a hyalinized stroma was found. In lesions of neoplasms of spindle cells histological studies should be supported by an immunohistochemical panel and show positive results to antibodies Actin, smooth muscle Actin and Vimentin.
El miofibroma es una neoplasia benigna compuesta por células mioides contráctiles localizadas alrededor de la pared de vasos sanguíneos delgados, es un tumor que se presenta sobre todo en la infancia aunque puede ocurrir a cualquier edad, tiene predilección en cabeza y cuello, sin embargo en cavidad oral es raro y aún más si es intraóseo, puede estar asociado a miofibromatosis o bien presentarse de manera solitaria. Los diagnósticos diferenciales dependen de la localización y de las características radiográficas y de primera instancia es muy difícil incluir al miofibroma entre las lesiones intraóseas. Histológicamente presenta patrón bifásico conformado por células fusiformes dispuestas en fascículos y haces así como núcleos fusiformes con citoplasma eosinófilo dentro de un estroma hialinizado. Es necesario recurrir al panel de inmunohistoquímica en neoplasias de células fusiformes, positivo a Acs Actina, Actina músculo liso y Vimentina. Reportamos el caso de una mujer de 45 años con un miofibroma en la mandíbula.
Sujets)
Humains , Femelle , Adulte d'âge moyen , Myofibrome/anatomopathologie , Myofibrome , Tumeurs de la mandibule/anatomopathologie , Tumeurs de la mandibule , ImmunohistochimieRésumé
O Dermatomiofibroma está incluído no grupo de lesões neoplásicas mesenquimais benignas de linhagem fibroblástica e miofibroblástica da pele. É uma doença rara, havendo aproximadamente 100 casos descritos na literatura mundial até o momento. Este artigo relata o caso de uma mulher jovem com apresentação clínica típica e diagnóstico histopatológico de dermatomiofibroma. Foram realizadas colorações especiais que mostraram preservação das fibras colágenas e a imunohistoquímica revelou positividade para vimentina e negatividade para actina e S100. Por se tratar de doença rara, os achados histopatológicos são de grande importância, mas a supeição clínica é possível em casos típicos como este.
Dermato myofibroma is included in the group of benign cutaneous mesenchymal neoplastic lesions of fibroblastic and myofibroblastic lineage. It's a rare disease and there are approximately only one hundred cases described worldwide in the medical literature up to now. The present study reports the case of a young woman with typical clinical cutaneous lesion and histopathological diagnosis of dermato myofibroma. Special stains were carried out which showed preserved collagen fibers and immunohistochemistry was positive for vimentin and negative for actin and S100. As it is a rare disease, the histopathological findings are of great importance but clinical suspicion is possible in typical cases such as this one.
Sujets)
Adolescent , Femelle , Humains , Myofibrome/anatomopathologie , Maladies rares/anatomopathologie , Tumeurs cutanées/anatomopathologie , Myofibroblastes/anatomopathologie , Coloration et marquageRésumé
Inflammatory myofibroblastic tumor (IMT) is a very rare benign mass lesion. Inflammatory myofibroblastic tumor of genitourinary tract most frequently involves the urinary bladder. Renal inflammatory myofibroblastic tumor is rare. Renal inflammatory myofibroblastic tumor usually remain asymtomatic until the tumor occurs obstructive uropathy. We experienced a case of right renal inflammatory myofibroblastic tumor.
Sujets)
Granulome à plasmocytes , Rein , Myofibroblastes , Myofibrome , Vessie urinaireRésumé
Myofibroma is a rare benign soft tissue tumor that in all ages usually occurs in the head and neck region, and at subcutaneous tissue, but rarely has bone origin within bone. Intraosseous lesions are more often found in childhood. Although intraosseous lesions are relatively common in mandible. Reports for mandible, reports on radiographic findings of myofibroma occurred on the mandible are uncommon. We describe the radiographic appearance on the conventional radiographs and CT of myofibroma of the mandible in a 9-year-old boy. This benign lesion closely resembles to odontogenic cyst or tumor on image.
Sujets)
Enfant , Humains , Mâle , Tête , Mandibule , Myofibrome , Cou , Kystes odontogènes , Tissu sous-cutanéRésumé
Solitary myofibroma is a recently described, uncommon neoplasm of superficial soft tissue and presenting as an asymptomatic nodule of the skin, which mainly occurs in adults. It was thought to be the adult counterpart of infantile myofibromatosis. Although it has histopathological similarity to infantile myofibromatosis, it shows a discrete clinicopathological entity compared with infantile form on the basis of its much later onset, superficial location, invariable solitary occurrence and uniformly benign behavior. A 41-year-old female presented a skin lesion on the posterior neck for 1 year, which was a solitary, painless nodule. The excised tumor was diagnosed as myofibroma by light microscopy and immunohistochemistry.
Sujets)
Adulte , Femelle , Humains , Immunohistochimie , Microscopie , Myofibrome , Myofibromatose , Cou , PeauRésumé
Solitary myofibroma is an uncommon myofibroblastic neoplasm, presenting as an asymptomatic nodule of the skin, which mainly occurrs in adults. It was thought to be the adult counterpart of infantile myofibromatosis. Although it has histopathological similarity to infantile myofibromatosis, it shows a discrete clinicopathological entity compared with infantile form on the basis of its much later onset, superficial location, invariable solitary occurrence and uniformly benign behavior. A-45-year-old woman had a skin lesion on the right upper eyelid for 2 years, which was a solitary, painless nodule with eyelid retraction. The excised tumor was diagnosed as myofibroma by light microscopy and immunohistochemistry.
Sujets)
Adulte , Femelle , Humains , Paupières , Immunohistochimie , Microscopie , Myofibroblastes , Myofibrome , Myofibromatose , PeauRésumé
Solitary myofibroma is a recently described, benign neoplasm of superficial soft tissue, which represents the adult counterpart of infantile myofibromatosis. In Korean literature, only one case has been reported, and we report an additional case of solitary myofibroma in a 57-year-old man. He presented with 2-month history of asymptomatic slightly elevated pale-skin-colored nodule occurring on the right side of philtrum. A histopathologic examination revealed diffuse proliferation of fibroblasts and spindle cells with a whorled arrangement and grouped fascicles. The tumor cells were positively stained for vimentin and smooth muscle actin.
Sujets)
Adulte , Humains , Adulte d'âge moyen , Actines , Fibroblastes , Immunohistochimie , Lèvre , Muscles lisses , Myofibrome , Myofibromatose , VimentineRésumé
Adult solitary cutaneous myofibroma is a recently described benign neoplasm of the skin or subcutis, representing the adult counterpart of infantile myofibroblastoma. The histologic and immunohistochemical features of a 21-year-old woman with a solitary brownish, mildly tender nodule on her right thigh are reported here. The nodule had been present for a duration of 3 years. It showed a nodular dermal mass with an irregular margin. The lesion consisted of interlacing bundles of spindle cells which were positive for smooth muscle actin, muscle specific actin and vimentin. Immunohistochemical stainings for desmin, S-100 protein, CD 34 and CD 68 were negative. Cutaneous myofibroma in an adult is a distinct entity of benign neoplasm.
Sujets)
Adulte , Femelle , Humains , Jeune adulte , Actines , Desmine , Muscles lisses , Myofibrome , Tumeurs du tissu musculaire , Protéines S100 , Peau , Cuisse , VimentineRésumé
Myofibroma and myofibromaiosis are uncommon, benign, mostly self-limiting, probably hamartomatous proliferation of myofibroblasts. These tumors are most often present at birth or before the age of 2 years with multiple ot solitary lesion. Adult cases are rare and most,ly showed as a solitary lesion. We present a case of solitary myofibroma that developed on the vertex of scalp in a 51-year-old man. The lesion was a mild tender and intermittent pruritic firm, pedunclated 1 1cm sized tumor. The iumor was diagnosed as myofibroma by light microscopy and immunohistochemistry.